Fedorov V D, Kubyshkin V A, Budaev K D
Khirurgiia (Mosk). 2003(7):38-42.
Generalized leiomyomatosis of the esophagus and stomach is a rare disease. According to literature data, age of the patients ranges from 9 to 84 years, in men this disease is diagnosed 2 times more often than in women. It is necessary to differentiate this disease with cancer, esophageal diverticulum, achalasia of the cardia, hiatal hernia. In our case a woman of 32 years was ill from 5 years of age. Correct diagnosis was not made before surgery. Endoscopic examination suspected leiomyoma. Only repeated urgent histological tests during surgery resulted in accurate diagnosis (subtotal leiomyomatosis of the esophagus and proximal stomach) and in adequate surgical policy--subtotal resection of the esophagus and proximal resection of the stomach with Toprover gastrostoma creation. Then, retrosternal esophagocoloplasty was performed. 2 years after the first surgery the state of the patients is satisfactory, there are no symptoms of recurrence of the disease.
食管和胃广泛性平滑肌瘤病是一种罕见疾病。根据文献资料,患者年龄从9岁至84岁不等,男性患此病的诊断率是女性的2倍。有必要将这种疾病与癌症、食管憩室、贲门失弛缓症、食管裂孔疝相鉴别。我们的病例是一位32岁女性,从5岁起患病。手术前未做出正确诊断。内镜检查怀疑为平滑肌瘤。仅在手术期间重复进行紧急组织学检查才得出准确诊断(食管和近端胃大部平滑肌瘤病)并制定了适当的手术策略——食管大部切除术和近端胃切除术并创建托普弗胃造口术。然后,进行了胸骨后食管结肠吻合术。首次手术后2年,患者状况良好,无疾病复发症状。